Haemophilia patients aged 0 - 18 years in the Western Cape

Objectives. To record the number of haemophiliacs aged 0- 18 years in the Western Cape (WC), what event led to the diagnosis, the level of clotting factor, treatment, functional status of their joints and impact of the disease on the family.Design. A prospective study of patients registered with the South African National Haemophilia Registry and new patients, utilising the patients' paediatricians, hospital records, patient and guardian interviews, physicalexamination and provincial nurse haemophilia co-ordinators.Setting. Haemophilia care centres at the three WC academic hospitals, regional hospitals and homes of patients. Two elective medical students, MHH and JJH, collected the information.Subjects. All boys with confirmed haemophilia A or B in the WC.Outcome measures. Events that led to diagnosis, degree of haemophilia, use of clotting factor, functional status, and effect on family.                                                                                                                                       Results. Of 78 patients (59 haemophilia A, 19 haemophilia B) identified, 49 could be studied. Forty-three per cent had severe, 29% moderate and 22% mild disease (6% unknown). Family history was present in 49%, but led to diagnosis in only 12%. The most common first symptoms were subcutaneous and mucosal bleeding. Delay in diagnosis varied from 0 to 9 months. Twenty-nine per cent of guardians were suspected of child abuse. RSA produced clotting factor was used 'on demand' in 73% of patients, for periodic prophylaxis in 20% and as continuous prophylaxis in 7%. Joints were functionally restricted in 43% of patients. The majority of guardians (59%) said the disease had a major impact on the family.Conclusions. The diagnosis of haemophilia in children with a positive family history was often delayed. Haemophilia causes significant morbidity in our patients and their families

Investigating the effect of sintering rate and solvent type on the liquid transport kinetics of α-alumina powder compacts

The ceramic materials' properties and the penetrating liquid both influence liquid transport into ceramic catalytic materials. Terahertz pulsed imaging (TPI) in combination with a flow cell was used to investigate the transport process of polar and less polar solvents into a range of α-alumina powder compacts. The TPI results show that the alumina samples with the largest heating rate (200 °C h−1) have the fastest water transport. The TPI results also reveal that 1-octanol takes much longer to transport through the alumina samples than water, as the viscosity of 1-octanol is much larger than that of water. Since 1-octanol is semi-transparent to terahertz radiation, it was possible to study the liquid transport process and the structural changes behind the liquid front, such as the change in the refractive index of the compact and the fill fraction of 1-octanol in the compact as a function of time

Terahertz pulsed imaging as a new method for investigating the liquid transport kinetics of α-alumina powder compacts

Investigating the liquid transport kinetics of solid catalysts is of great importance for gaining a better understanding of the manufacturing and performance of such catalysts during reaction upon contact with the liquid. Terahertz pulsed imaging (TPI) coupled with a newly designed flow cell was used to quantify the rate of water ingress into α-alumina pellets with a range of different porosities. A wide range of compaction forces (cold compaction, 7–58 kN) and sintering conditions (no firing and sintering at 1200 and 1300 °C) was investigated to explore the optimal pellet microstructure, i.e. mechanically strong but sufficiently porous for fast liquid transport kinetics. The results confirm that both the microstructure characteristics, particularly porosity, as well as the surface properties, i.e. wettability, influence the liquid transport kinetics. Fitting the TPI penetration rates with a power law shows that the type of observed mass transport characteristics is consistent with Darcy flow. The Lucas–Washburn equation was used to calculate the hydraulic radius based on the transport data. In summary, the results demonstrate that TPI has great potential to study the liquid transport kinetics of porous ceramic catalysts and catalyst supports and that can comfortably quantify transport processes at rates of 250 μm s−1 and beyond in such substrates for better quality control and optimised design and performance of such materials

Observation of the 0+ 2 and γ bands in 98Ru, and shape coexistence in the Ru isotopes

Excited states in 98Ru were investigated using γ-ray spectroscopy following the β-decay of 98Rh, and via the 100Ru(p,t) reaction. Combining the results from the two experiments, two states were revised to have spin-parity of 4+ and subsequently assigned to the 02+ and “γ” bands, respectively. The observed structures in 98Ru are suggested to be deformed and rotational, rather than spherical and vibrational, and fit well into the systematics of these excitations in the Ru isotopes. The 02+ excitation is suggested as a shape coexisting configuration. This observation eliminates some of the last remaining candidates for nearly harmonic vibrational nuclei in the Z≈50 region. Beyond-mean-field calculations are presented that support shape coexistence throughout the Ru isotopes with N=52–62, and suggest a smooth evolution of the shape

Investigation of excited 0+ states in 160Er populated via the (p, t) two-neutron transfer reaction

Many efforts have been made in nuclear structure physics to interpret the nature of low-lying excited 0+ states in well-deformed rare-earth nuclei. However, one of the difficulties in resolving the nature of these states is that there is a paucity of data. In this work, excited 0+ states in the N = 92 nucleus 160Er were studied via the 162Er(p, t)160Er two-neutron transfer reaction, which is ideal for probing 0+ → 0+ transitions, at the Maier-Leibnitz-Laboratorium in Garching, Germany. Reaction products were momentum-analyzed with a Quadrupole-3-Dipole magnetic spectrograph. The 0+2 state was observed to be strongly populated with 18% of the ground state strength

H-1 nmr as a non-invasive probe of amniotic fluid in insulin dependant diabetes mellitus

Ten amniotic fluid samples obtained from third trimester pregnant women suffering from insulin dependant diabetes mellitus were analysed by 1H-NMR and compared to ten samples from a group of normal volunteers. A subset of the metabolites identified; valine, lactate, alanine, acetate, citrate and glucose were quantitated using standard addition methods. Apart from valine and citrate, a general diminution in the concentration of each of these species was found, especially glucose, in the diabetic group. The abnormally low glucose levels in the diabetic group are suggestive of infection in the patient group. However, the depressed lactate levels in the diabetic group suggest that in these cases the fetus was not subjected to stress